Chronic mast cell leukemia: A novel leukemia-variant with distinct morphological and clinical features

Valent, Peter; Sotlar, Karl; Sperr, Wolfgang R.; Reiter, Andreas; Arock, Michel; Horny, Hans‐Peter

doi:10.1016/j.leukres.2014.09.010

Cited by 92 publications

(63 citation statements)

References 43 publications

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“…Chronic MCL is defined by mature mast cell morphology and an indolent course.Mast cells in MCL tend to be immature and lose the spindle shape conventionally seen in ISM.Morphologic variants include a spectrum from blast‐like, round to spindle‐shaped cells with varying degrees of granularity. Some forms may have coarse purple granules (Georgin‐Lavialle et al, ; Valent et al, ; Swerdlow et al, ).Patients with MCL have the poorest overall survival (Pardanani et al, ) and should be considered for trials using newer targeted agents, high dose chemotherapy and bone marrow transplant if clinically eligible.…”

Section: Smouldering Systemic Mastocytosis (Ssm)mentioning

confidence: 99%

The clinical and pathological panoply of systemic mastocytosis

Radia¹,

Gréen

Oni³

et al. 2020

Br J Haematol

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Summary Mastocytosis is a rare disease with varied presentation, myriad symptomatology and variable prognosis. Most patients present with cutaneous disease and mediator‐related symptomatology with a small subset having systemic disease (systemic mastocytosis, SM). A subset of the latter develops synchronous or metachronous haematologic neoplasms (SM‐AHN), most commonly chronic myelomonocytic leukaemia (CMML). Advanced systemic mastocytosis (ASM) is seen in a relatively small number of patients and is usually associated with organ dysfunction, and may present with hepatosplenomegaly, lymphadenopathy and ascites with progression to leukaemic transformation (mast cell leukaemia/acute myeloid leukaemia) occurring in a few patients. This paper discusses the clinical and pathologic features of the entire spectrum of SM in adults.

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Section: Smouldering Systemic Mastocytosis (Ssm)mentioning

confidence: 99%

The clinical and pathological panoply of systemic mastocytosis

Radia¹,

Gréen

Oni³

et al. 2020

Br J Haematol

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“…However, few patients experience a rather chronic clinical course coupled with a more mature mast cell proliferation. For these cases the definition of “chronic MCL” has been proposed …”

Section: Mastocytosismentioning

confidence: 99%

Update on the classification of myeloid neoplasms: The 2016 revised World Health Organization classification of hematopoietic and lymphoid neoplasms

Sangiorgio

Orazi

2019

Adv Cell Gene Ther

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In recent years, a large body of knowledge regarding the molecular genetics of myeloid malignancies has emerged and molecular findings have become increasingly important not only for diagnosis but also to establish prognosis and treatment of patients with myeloid malignancies. Clinical and pathological findings have been refined in relation to their diagnostic/prognostic value. The integration of all these different parameters represents the backbone of the 2016 revised 4th Edition of the WHO Classification of Tumours of Hematopoietic and Lymphoid Tissues. The current review aims to highlight the main changes between the 2008 WHO Classification and the most recent 2016 revised 4th edition, with regard to the classification of myeloid malignancies. K E Y W O R D S Acute myeloid leukemia, myelodysplastic syndromes, myeloproliferative neoplasms, WHO classification 2 of 9 | SANGIORGIO ANd ORAZI 2.2 | BCR-ABL1 negative myeloproliferative neoplasms | Polycythemia veraMajor changes in the clinical criteria for the diagnosis of polycythemia vera (PV) have been adopted in the revised classification. Hemoglobin (Hb) >18.5 g/dL for men and > 16.5 g/dL for women were the thresholds used to diagnose PV in the 2008 classification. Applying these criteria, many cases of early phase PV were left unrecognized, for example, patients with a red cell mass > 25% of the mean predicted value (thus indicative of increased red cell volume), but with Hb level falling below the stated thresholds. For such cases, the definition of "masked PV" has been introduced. 7,8 Similarly, application of the 2008 criteria can lead to misdiagnose as essential thrombocythemia (ET) cases of early PV presenting with thrombocytosis. 9,10 To address these issues, the revised classification has established Hb > 16.5 for men and > 16.0 for women as the new thresholds for the diagnosis of PV. The newer TA B L E 1 Summary of the major diagnostic changes introduced by the 2016 WHO classification, revised 4th edition

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“…30 These patients have stable serum tryptase levels, lack end-organ damage and appear to have a less aggressive course with symptoms and progression controlled without chemotherapy. A subset of patients have progressed to conventional/acute MCL.…”

Section: Mast Cell Leukaemiamentioning

confidence: 99%

The bone marrow in systemic mastocytosis – an update

Moonim

2015

Diagnostic Histopathology

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Chronic mast cell leukemia: A novel leukemia-variant with distinct morphological and clinical features

Cited by 92 publications

References 43 publications

The clinical and pathological panoply of systemic mastocytosis

The clinical and pathological panoply of systemic mastocytosis

Update on the classification of myeloid neoplasms: The 2016 revised World Health Organization classification of hematopoietic and lymphoid neoplasms

The bone marrow in systemic mastocytosis – an update

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